Application of the 2021 EAN/PNS criteria for chronic inflammatory demyelinating polyneuropathy.

BACKGROUND: The diagnostic value of new criteria of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) for chronic inflammatory demyelinating polyneuropathy (CIDP) is unknown. METHODS: We performed a retrospective study of fulfilment of EAN/PNS 2021 criteria on 120 consecutive patients with a clinical diagnosis of 'suspected CIDP' and objective treatment response, attending University Hospitals Birmingham, UK. Specificity was evaluated versus 100 consecutive controls. RESULTS: The sensitivity of EAN/PNS criteria for 'CIDP' was 83.3%. The sensitivity for 'CIDP' or 'possible CIDP' was 93.3%. Specificity was of 94% for 'CIDP' and 79% for 'CIDP' or 'possible CIDP'. No sensitivity/specificity differences were ascertained with previous versions ('CIDP': sensitivity: 83.3% vs 81.3%, p=0.74, specificity: 94% vs 96.1%, p=0.38, respectively; 'CIDP' or 'possible CIDP': sensitivity: 93.3% vs 96.7%, p=0.25 and specificity: 79% vs 69.2 %, p=0.09, respectively). F-wave prolongation, proximal and distal temporal dispersion were the most likely parameters to contribute to false positives, whereas distal motor latency was the least likely. No impact of sensory electrophysiology could be ascertained. 'Typical CIDP' represented 79% of the CIDP cohort. The largest component of the 'variant CIDP' group was represented by focal/multifocal forms (14%). With new criteria, 6.7% of the cohort did not meet requirements, among whom the majority (75%) had paranodopathy or chronic immune sensory polyradiculopathy (CISP). DISCUSSION: The sensitivity and specificity of new EAN/PNS criteria for CIDP is equivalent to that of previous versions. The exclusion of paranodopathies and CISP from the CIDP spectrum impacts on management of a non-negligible proportion of treatment-responsive patients.

Causes and consequences of diagnostic delay in Guillain-Barré syndrome in a UK tertiary center.

INTRODUCTION/AIMS: Understanding the potential causes and consequences of diagnostic delay in Guillain-Barré syndrome (GBS) could improve quality of care and outcomes. We aimed to determine these causes and consequences in our cohort of patients with GBS. METHODS: We retrospectively reviewed records of subjects with GBS, admitted to our center at University Hospitals Birmingham, UK, between January 2005 and December 2020. We evaluated time to diagnosis from presentation, factors associated with diagnostic delay, and its potential consequences. RESULTS: We included 119 consecutive subjects. Diagnostic delay at least 5 days from first presentation occurred in 27 of 119 (22.7%) of patients. Diagnostic delay was associated with age >60 years (odds ratio [OR], 3.58; 95% confidence interval [CI], 1.44-8.85), pre-existing cardiac/respiratory disease (OR, 4.10; 95% CI, 1.46-11.54), pre-existing diabetes (OR, 10.38; 95% CI, 2.47-43.69), documented normal initial neurological examination (OR, 2.49; 95% CI, 1.03-6.02), initial assessment by primary care (OR, 3.33; 95% CI, 1.22-9.10) and at least one visit for medical attention (OR, 10.29; 95% CI, 3.81-27.77). Diagnostic delay was not associated with length of inpatient stay, intensive care unit admission, ventilation, ability to walk at discharge, or inpatient mortality. Independent associations with diagnostic delay were observed for at least one visit for medical attention (OR, 10.15; 95% CI, 3.64-28.32) and pre-existing cardiac/respiratory disease (OR, 3.98; 95% CI, 1.19-13.28). An association of diagnostic delay with inpatient mortality was ascertained specifically in subjects with classic GBS (OR, 5.33; 95% CI, 1.1-25.87). DISCUSSION: Diagnostic delay in GBS results from patient-specific factors and patient pathways. A high index of suspicion is appropriate for certain patient groups. Prospective studies are needed to further investigate this topic.

Acute-onset polyradiculoneuropathy after SARS-CoV2 vaccine in the West and North Midlands, United Kingdom.

INTRODUCTION/AIMS: We aimed to determine whether specific severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) vaccines may be associated with acute-onset polyradiculoneuropathy and if they may result in particular clinical presentations. METHODS: We retrospectively reviewed records of all persons presenting with acute-onset polyradiculoneuropathy from January 1, 2021, to June 30, 2021, admitted to two Neuroscience centers, of the West and North Midlands, United Kingdom. We compared subjects with previous SARS-CoV2 vaccine exposure with a local cohort of persons with acute-onset polyradiculoneuropathy admitted between 2005 and 2019 and compared admission numbers for the studied time frame with that of the previous 3 years. RESULTS: Of 24 persons with acute-onset polyradiculoneuropathy, 16 (66.7%) presented within 4 weeks after first SARS-CoV2 vaccine. Fourteen had received the AstraZeneca vaccine and one each, the Pfizer and Moderna vaccines. The final diagnosis was Guillain-Barré syndrome (GBS) in 12 and acute-onset chronic inflammatory demyelinating polyneuropathy in 4. Among AstraZeneca vaccine recipients, facial weakness in nine persons (64.3%), bulbar weakness in seven (50%), and the bifacial weakness and distal paresthesias GBS variant in three (21.4%), were more common than in historical controls (P = .01; P = .004, and P = .002, respectively). A 2.6-fold (95% confidence interval: 1.98-3.51) increase in admissions for acute-onset polyradiculoneuropathy was noted during the studied time frame, compared to the same period in the previous 3 years. DISCUSSION: Despite a low risk, smaller than that of SARS-CoV2 infection and its complications, exposure to the first dose of AstraZeneca SARS-CoV2 vaccine may be a risk factor for acute-onset polyradiculoneuropathy, characterized by more common cranial nerve involvement.

A patient with massive cerebral arterial air embolism

@#Cerebral air embolism is potentially a catastrophic event that occurs as a consequence of air entry into the vasculature. We report a mechanically ventilated 72-year-old woman who underwent multiple procedures during intensive care stay with few possible sources of emboli postulated. We also discuss regarding the preventive measures to minimise the risk of air embolism.